【摘要】：目的探討Van Wyk-Grumbach綜合征(VWGS)的早期診斷和治療。方法回顧分析1例VWGS患兒的臨床資料,并復(fù)習(xí)相關(guān)文獻(xiàn)。結(jié)果患兒,女,9歲,因身高增長(zhǎng)緩慢、肥胖3年,伴乳房增大6個(gè)月、陰道出血3個(gè)月就診。游離甲狀腺素(FT4)0.46 ng/d L,促甲狀腺素(TSH)150 m IU/L;GnRH激發(fā)試驗(yàn)提示性腺軸未啟動(dòng);血泌乳素、雌二醇明顯升高;骨齡延遲;彩色超聲示子宮、卵巢增大,可見(jiàn)卵巢囊腫;垂體MRI示腺垂體增生。予左甲狀腺素鈉治療2個(gè)月后甲狀腺功能恢復(fù)正常,復(fù)查彩超示雙卵巢明顯縮小,體質(zhì)量減輕6 kg,未再陰道出血;3個(gè)月后雙卵巢至正常大小,鞍區(qū)MRI占位縮小;6個(gè)月后復(fù)查彩色超聲示子宮、卵巢大小正常,無(wú)卵巢囊腫,鞍區(qū)MRI占位消失。結(jié)論 VWGS是長(zhǎng)期未經(jīng)治療的嚴(yán)重原發(fā)性甲狀腺功能減退癥少見(jiàn)的并發(fā)癥,好發(fā)于青春期女童,甲狀腺素替代治療有效。
[Abstract]:Objective to investigate the early diagnosis and treatment of Van Wyk-Grumbach syndrome (VWGS). Methods the clinical data of one case with VWGS were retrospectively analyzed and the related literature was reviewed. Results the female, 9 years old, had a slow increase in height, obesity for 3 years, breast enlargement for 6 months and vaginal bleeding for 3 months. Free thyroxine (FT4) 0.46 ng/d / L, thyrotropin (TSH) 150m IU/L;GnRH excitation test showed that gonadal axis was not activated, serum prolactin and estradiol were significantly increased, bone age was delayed, and serum prolactin and estradiol were significantly increased. Color ultrasound showed uterus, ovarian enlargement, ovarian cyst, pituitary MRI showed adenohypophysis hyperplasia. The thyroid function returned to normal after 2 months of treatment with levathyroxine sodium. The color Doppler ultrasound showed that the diovaries were obviously reduced and the body mass was reduced for 6 kg, without vaginal bleeding, and after 3 months the size of the two ovaries reached the normal size and the MRI occupied the Sellar region decreased. After 6 months, color ultrasound showed normal ovarian size, no ovarian cyst, and MRI space occupying in Sellar region disappeared. Conclusion VWGS is a rare complication of severe primary hypothyroidism without treatment for a long time. It is more common in adolescent girls. Thyroxine replacement therapy is effective.
【作者單位】： 江西省兒童醫(yī)院內(nèi)分泌遺傳代謝科;
【分類號(hào)】：R725.8

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